Gliederung

Objective: In prospective randomized studies deep brain stimulation (DBS) of the globus pallidus internus (Gpi) has been shown to be effective in adult patients with idiopathic generalized or segmental dystonia. Early onset idiopathic generalised dystonia is a progressive and disabling condition with loss of ambulation, speech and difficulties with feeding. We describe results of deep brain stimulation of the Gpi in three children and eight young adults with early-onset generalized dystonia.

Methods: Three children (mean age 12.3 years, range 9–14 years) and eight young adults (mean age 21.6 years, range 19–25 years) underwent bilateral DBS of the Gpi due to failure of medical treatment for generalized dystonia. Mean duration of the disease was 10.6 years (range 5–14 years) in children and 17 years (range 10–21 years) in young adults. The complete procedure was performed under general anaesthesia, including stereotactic MRI, electrophysiological mapping of the Gpi, intraoperative test stimulation to determine thresholds for stimulation-induced adverse effects, and implantation of the pulse generator.

Results: The overall clinical improvement from baseline was in mean 68.86% (range 20.39 to 98.52%) after in mean 28 months (range 6–48 months) according to the Burke-Fahn-Marsden-Dystonia-Rating-Scale (BFMDRS). In one patient postoperative MRI showed a symptomatic infarction of the Gpi with a reversible paresis and an asymptomatic small haemorrhage in the contralateral Gpi.

Conclusions: Deep brain stimulation of the Gpi is an effective treatment also for childhood-onset dystonia. Severe secondary problems, such as irreversible orthopaedic defective positions or psychosocial mental retardation can be avoided by an earlier intervention.